Snapshot A 21-year-old gentleman comes to the emergency room with a painful rash all over his body, including some lesions in his mouth. He also describes feeling feverish. On physical exam, his skin has multiple bullae that sloughs off easily with a single rub. The rash covers > 30% of his body. A careful history reveals that he was recently put on lamotrigine for his epilepsy. The lamotrigine is stopped and patient is immediately admitted to the burn unit. Introduction Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – two diseases on the same spectrum SJS: < 10% of body surface area TEN: > 30% of body surface area SJS/TEN overlap: 10-30% of body surface area Severe, febrile blistering disease of skin and mucous membranes often caused by drugs (>>> infection) e.g., penicillin, sulfonamides, phenytoin, carbamazepine, lamotrigine, NSAIDs can be caused by infection e.g., mycoplasma pneumonia Erythema multiforme (EM) is a distinct disease from SJS/TEN according to the current consensus definition Presentation Symptoms very painful skin (vs in EM, where pain/burning is typically very mild) systemic signs fever dehydration hypotension Physical exam initially dusky red macules or patches (not raised)that progress to tense bullae and eventual skin sloughing (vs in EM, where lesions are typically papular) mucous membranes always involved bullae and erosions in oral, genital, anal mucosa + Nikolsky sign (rubbing of skin easily causes sloughing – splitting of epidermis from dermis) Evaluation Based on clinical history and symptoms Skin biopsy: mainly to distinguish staphylococcal scalded skin syndrome and TEN full-thickness epidermal necrosis Labs: normal Differential Diagnosis Staphylococcal scalded skin syndrome Graft versus host disease Pemphigus vulgaris Erythema multiforme Treatment Discontinue causative agent Supportive care wound care fluids, electrolytes, nutrition Treat underlying infection Prognosis, Prevention, and Complications High mortality, especially with TEN